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Epidemiological Study of Hereditary Hemolytic Anemia in the Korean Pediatric Population during 1997-2016: a Nationwide Retrospective Cohort Study

Authors
Shim, Ye JeeJung, Hye LimShin, Hee YoungKang, Hyoung JinChoi, Jung YoonHah, Jeong OkLee, Jae MinLim, Young TakYang, Eu JeenBaek, Hee JoChoi, Hyoung SooYoo, Keon HeePark, Jun EunKim, SeongkooKim, Ji YoonPark, Eun SilIm, Ho JoonChueh, Hee WonKim, Soon KiLee, Jae HeeYoo, Eun SunPark, Hyeon JinLee, Jun AhPark, MeerimKang, Hyun SikPark, Ji KyoungLee, Na HeePark, Sang KyuLee, Young-HoLee, Seong WookChoi, Eun JinKong, Seom Gim
Issue Date
24-8월-2020
Publisher
KOREAN ACAD MEDICAL SCIENCES
Keywords
Congenital Hemolytic Anemia; Hereditary Spherocytosis; Hemoglobinopathies; Thalassemia; Glucose-6-phosphate Dehydrogenase Deficiency; Pyruvate Kinase
Citation
JOURNAL OF KOREAN MEDICAL SCIENCE, v.35, no.33
Indexed
SCIE
SCOPUS
KCI
Journal Title
JOURNAL OF KOREAN MEDICAL SCIENCE
Volume
35
Number
33
URI
https://scholar.korea.ac.kr/handle/2021.sw.korea/130535
DOI
10.3346/jkms.2020.35.e279
ISSN
1011-8934
Abstract
Background: Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA. Methods: We collected the data of a newly diagnosed pediatric HHA cohort (2007-2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997-2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey. Results: A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia. Conclusion: In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.
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